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        Atypical Fibroxanthoma

 
 

         

Central carcinoid tumour is the most common type and usually presents as a slow-growing, solitary,  polypoid mass, less than 3 to 4 cm in diameter, within a major bronchus.  Image Link

They may infiltrate the bronchial wall and extend to the surrounding parenchyma and even reach the pleura or myocardium.

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Microscopically, they are covered by bronchial epithelium and are composed of uniform small, round to polygonal cells arrayed in well-defined nests or cords separated by thin fibrovascular septa ( "Zellballen" ).

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Other growth patterns include serpiginous cords or formation of small, rosette-like microacinar structure.

Cytologically, the tumor cells contain abundant lightly eosinophilic, granular cytoplasm with central nuclei.

The nuclei usually display a scattered, coarse ("salt-and-pepper" stippling of chromatin and an occasional nucleolus.

Mitotic figures are absent or rarely seen.

Unusual variants are with oncocytic cytoplasm (oncocytic carcinoid), with melanin pigment (pigmented carcinoid) and carcinoid with metaplastic bone formation, gland formation and abundant production of stromal mucins. A variant composed predominantly of spindle cells arranged in fascicular pattern resembling sarcoma (usually peripheral type).

Histochemically, they are argentaffin-negative and argyrophil-positive.

Immunohistochemical stains show positive reactivity for broad-spectrum and low molecular weight cytokeratin, chromogranin, synaptophysin, neuro-specific enolase, serotonin, bombesin etc.

Electron microscopy demonstrates dense-core neurosecretory granules.

Oncocytic carcinoid is a variant of carcinoid tumor in which the tumour cells have an abundant, granular acidophilic cytoplasm. Their behavior is similar to that of central carcinoid.

                 

Cytopathology of oncocytic carcinoid tumor of the lung mimicking granular cell tumor. A case report.Acta Cytol. 2000 Mar-Apr;44(2):247-50.

BACKGROUND: The cytopathologic features of oncocytic carcinoid tumor of the lung, a rare variant of carcinoid tumor that is composed exclusively of oncocytes, have not been described before in detail. CASE: The bronchial brush smears from an 80-year-old female with an endobronchial obstructive tumor showed single and loose clusters of tumor cells with abundant granular, eosinophilic cytoplasm. The differential diagnoses included oncocytic carcinoid tumor, granular cell tumor, other oncocytic tumors of bronchial origin and metastatic oncocytic tumors. Immunocytochemistry and electron microscopy confirmed the diagnosis of oncocytic carcinoid tumor. CONCLUSION: Oncocytic carcinoid tumor of the lung has cytopathologic features similar to those of granular cell tumor and pulmonary oncocytoma. Immunocytochemistry, electron microscope or both are necessary to distinguish these neoplasms.

Pigmented pulmonary carcinoid tumor. An immunohistochemical and ultrastructural study. Arch Pathol Lab Med. 1993 Aug;117(8):832-6.

We evaluated three cases of pigmented pulmonary carcinoid tumors that were retrieved from the files of the Armed Forces Institute of Pathology, Washington, DC. Clinical follow-up showed no indication of tumor recurrence or metastases, nor was there evidence of malignant melanoma. All three cases exhibited histologic features of typical carcinoid tumor; there were focal oncocytic changes in two cases. Finely dispersed, brown pigment, believed to be melanin, was distributed in two different patterns: in sustentacular cells (case 1) or within the tumor cells (cases 2 and 3). Fontana-Masson stain was positive in areas of this pigment in all cases. The tumor cells showed immunoreactivity for chromogranin, synaptophysin, keratin (AE1/AE3 and CAM-5.2), and S100 protein in all cases. Focal staining for vimentin and corticotropin was seen within neoplastic cells in two cases. The pigmented sustentacular cells in case 1 showed focal immunoreactivity for S100 protein and HMB-45. Ultrastructural studies of paraffin-embedded tissues were performed in two cases. They showed well-developed melanosomes in the pigmented sustentacular cells in case 1. In both cases, cytoplasmic neurosecretory-type granules were identified in neoplastic cells. These findings demonstrate that pigmented pulmonary carcinoid tumor has an immunohistochemical profile similar to that of typical pulmonary carcinoid tumor. In some instances, pigmented pulmonary carcinoid tumors may show ultrastructural evidence of melanocytic and neuroendocrine differentiation. These immunohistologic and ultrastructural findings distinguish pigmented pulmonary carcinoid tumor from malignant melanoma and support the concept of "multidirectional cellular differentiation."

Oncocytic carcinoid of the lung.Radiat Med. 1993 Mar-Apr;11(2):63-5.

The case of a 47-year-old man with an oncocytic variant of carcinoid of the lung is reported. Chest radiography disclosed a well-defined round mass in the left upper lobe of the lung. Contrast-enhanced CT demonstrated a homogeneously well-enhanced mass. The oncocytic variant of a typical carcinoid is a rare histological entity, which can be established only by the ultrastructural features of mitochondrial hyperplasia and membrane-bound electron dense granules. Therefore, the definite diagnosis of oncocytic carcinoid tumor necessitates ultrastructural observation.

The histological spectrum of bronchial carcinoid tumours.Appl Pathol. 1989;7(4):205-18.

Sixty-three bronchial tumours are described. Using the criteria of necrosis, a mitotic count of 5 or greater per 10 high-power fields, well marked nuclear pleomorphism, lymphatic and vascular invasion, either singly or in combination, 38% of cases were considered atypical or well-differentiated neuroendocrine carcinoma. If an undifferentiated growth pattern was added as a further criterion, 49% of bronchial carcinoids were atypical. Oncocytic change was common (59% of cases) but only one pure oncocytic carcinoid was present in the series. Bone was seen in 25% of cases. In some cases it arose from the bronchial cartilage but in others it was seen in the centre of the tumour. The range of histological patterns seen in carcinoids is described. Rare types of bronchial carcinoids showed a papillary pattern as well as spindle cell foci. Two cell types were noted--large cells with eosinophilic cytoplasm and cells with small hyperchromatic nuclei and little cytoplasm. These cells are apoptotic as shown ultrastructurally. The differential diagnosis of these tumours is discussed. In 35 cases a clinico-pathological correlation was possible. Bronchial carcinoids are biologically unpredictable tumours. The present study indicates that nearly half the cases could be classified as atypical or well-differentiated neuroendocrine carcinoma. However, there still remained some cases where the histology was typical but subsequent lymph node metastases developed.

Pulmonary oncocytic carcinoid--a case report. Zhonghua Zhong Liu Za Zhi. 1985 Sep;7(5):394-5.

A pulmonary oncocytic carcinoid arising from the medial basal segment of the right lung is presented. The abundant oncocytic granules in the cytoplasm were observed by light-microscope. The ultrastructure by electronmicroscope showed that these granules originated from the hyperplasia of mitochondria, and there were membrane-bordered electric dense core granules (neuro-secretive granule) in the cytoplasm of this tumor. According to the morphological features observed by light and electronmicroscope, this tumor is considered as a subtype of bronchial carcinoid and belongs to the APUD system which should be differentiated from the metastatic tumor, the granular cell myoblastoma and the chemodectoma of lung.

Bronchial carcinoid tumors of the thorax: spectrum of radiologic findings. Radiographics. 2002 Mar-Apr;22(2):351-65.

Bronchial carcinoid tumors are neuroendocrine neoplasms that range from low-grade typical carcinoids to more aggressive atypical carcinoids and therefore demonstrate a wide spectrum of clinical behaviors and histologic features. Typical and atypical bronchial carcinoids have similar imaging features. Because most bronchial carcinoids are located in central airways, radiologic findings are usually related to bronchial obstruction. Central bronchial carcinoids manifest as an endobronchial nodule or hilar or perihilar mass with a close anatomic relationship to the bronchus. The mass is usually a well-defined, round or ovoid lesion and may be slightly lobulated at radiography and computed tomography (CT). Associated atelectasis, air trapping, obstructing pneumonitis, and mucoid impaction may also be seen. Peripheral bronchial carcinoids appear as solitary nodules. Calcification is common and is easily visualized at CT. Bronchial carcinoids demonstrate high signal intensity on T2-weighted and short-inversion-time inversion recovery magnetic resonance images. Prognosis of bronchial carcinoids is highly dependent on histologic findings: Atypical carcinoids have certain features that suggest a more aggressive nature. Typical bronchial carcinoids generally have an excellent prognosis, whereas atypical bronchial carcinoids have a worse prognosis. Therefore, understanding the histologic, clinical, and radiologic features of bronchial carcinoids facilitates accurate diagnosis and helps optimize surgical planning.

Video-assisted thoracic surgery (VATS) lobectomy for typical bronchopulmonary carcinoid tumors.Surg Endosc. 2000 Dec;14(12):1142-5.

BACKGROUND: Indications for the use of video-assisted thoracic surgery (VATS) lobectomy are a controversial matter. This study aims to provide a retrospective evaluation of VATS lobectomy in typical bronchopulmonary carcinoids. METHODS: Patient selection criteria for VATS lobectomy were as follows: (a) typical carcinoids with clear diagnosis; (b) centrally located lung tumors not amenable to bronchial resection with bronchoplastic procedures, or tumors located in peripheral lung tissues; (c) no hilar or mediastinal lymph node enlargement; and (d) normal respiratory function. Between January 1995 and December 1999, 12 patients (eight men and four women with a mean age of 57 years) were treated, seven with a peripheral and five with a centrally located tumor. Preoperative examination included chest roentgenograms, computed tomography (CT) of the chest, bronchoscopy, and spirometry; diagnosis was established by direct bronchoscopy in five cases, transbronchial biopsy in two cases, transthoracic biopsy in two cases, and video thoracoscopic wedge resection in three cases. Eleven VATS lobectomies and one VATS bilobectomy were performed. All patients underwent hilar lymphadenectomy and mediastinal sampling. RESULTS: There were no intraoperative complications. The only postoperative complication, hematothorax (8.3%), required VATS reoperation. Mean postoperative hospital stay was 5.33 days. Pathological examination of the resected specimens confirmed that the procedure was radical in all 12 patients and revealed eight T1N0 and four T2N0. At a mean follow-up of 30 months, no signs of recurrence were recorded. CONCLUSION: VATS lobectomy in the treatment of selected typical carcinoids, both central and peripheral, seems to yield favorable results and is therefore preferable to thoracotomy since it is less invasive.

Bronchial carcinoids. Our experience with 35 cases.Minerva Chir. 2000 Mar;55(3):113-9.

BACKGROUND: The aim of this study was to review personal experience regarding bronchial carcinoids. METHODS: This study investigated retrospectively 35 patients with bronchial carcinoids treated in our institution (Unit of General and Thoracic Surgery, University of Bologna) in 20 years (from 1978 to 1997). RESULTS: The m/f rate was 0.6, the average age 42.5 years and the smokers' percentage 42.8. The patients were symptomatic in 88.6% of cases. The carcinoid was located in the right lung in 17 patients (48.5%) and central in 29 patients (82.8%). Surgical treatment included pneumonectomy (6), lobectomy (17), bilobectomy (3), sleeve lobectomy (2), sleeve bilobectomy (1), sleeve resection of main bronchus (1), bronchotomy and tumorectomy (3) and wedge parenchymal resection (2). Thirty patients (85.7%) presented a typical carcinoid and five (14.3%) atypical carcinoid. Peribronchial and/or hilar lymphonodal metastases were present at surgery in 2 cases (5.7%), both centrally located and atypical. The typical carcinoids showed a real 5 years survival rate of 95.8% (with only one death, not related to the neoplasm), while that of the atypical carcinoids was 80% (one patient died of multiple metastases). CONCLUSIONS: The conclusions is drawn that although the carcinoid tumours are a distinct group of neuroendocrine lung neoplasms with a good prognosis in the majority of the cases, lobectomy and sleeve lobectomy are still the standard resection procedure for the majority of carcinoids. For atypical carcinoids lobectomies are the minimal oncologic surgical treatment.

                   

 
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